Obstructive jaundice often occurs as a late manifestation of non-Hodgkin's lymphoma (NHL), but has rarely been reported as a presenting manifestation, especially in children. We report a case of a 4-year-old boy with Burkitt's lymphoma (small non-cleaved cell NHL) who presented with obstructive jaundice, resulting from encasement of the common bile duct by the tumor. The patient underwent near-total excision of the tumor and biliary-enteric bypass to relieve the jaundice. Combined chemotherapy was not given because of refusal by his family. Two weeks after the operation, obstructive jaundice reappeared due to a large recurrent tumor compressing the liver hilum. He received chemotherapy and the jaundice disappeared within 6 days. Follow-up computed tomography 1 year later revealed total resolution of the tumor. Three conclusions are suggested by this case. First, although NHL presenting as obstructive jaundice is uncommon in children, it should be included in the differential diagnosis. Second, relief of obstructive jaundice can be effectively accomplished by chemotherapy alone. Third, chemotherapy should be given once NHL is diagnosed.